Dataset Information

Low VWF levels in children and lack of association with bleeding in children undergoing tonsillectomy.

ABSTRACT: von Willebrand disease is a common bleeding disorder, but diagnosis can be difficult in young children who have not had bleeding challenges. We sought to evaluate the correlation between bleeding and von Willebrand factor (VWF) levels in children undergoing surgical challenge with tonsillectomy. Children ages 0 to 18 undergoing tonsillectomy without a personal or family history of bleeding were enrolled prospectively following informed consent and institutional review board approval. VWF levels were obtained at the time of surgery. VWF antigen (VWF:Ag) and VWF activity (VWF:GPIbM) were tested via enzyme-linked immunosorbent assay. Bleeding score was calculated using the International Society of Hematology bleeding assessment tool (BAT). Surgical and postoperative bleeding were determined using questionnaires filled out by the surgeon and patient/family. A total of 1399 subjects were enrolled with evaluable data, with a median age of 5 years. The median VWF:Ag was 85 IU/dL and the median VWF:GPIbM was 100 U/dL. Median BAT for the entire population was 0, including those with postoperative bleeding. There was no difference in VWF level between those who experienced postoperative bleeding and those who did not, with median VWF:Ag 85 vs 85 (P = .89) and mean VWF:GPIbM 98 vs 100 (P = .5). Interestingly, there was a difference in VWF levels with age, with median VWF:Ag 81 for those younger than 3 years, 82 for those 3 to 6 years, 90 for those 7 to 10 years, and 100 for those 11 to 18 years. A similar trend was noted for VWF:GPIbM. Of the 2 to 6 year olds, 5% had VWF:Ag <50, which would meet criteria for low VWF, but only 1.8% had an abnormal BAT at study entry and only 2.5% bled after surgery. Only 1 subject with low VWF had an elevated postoperative BAT >2. These data suggest that low VWF levels do not correlate with bleeding in children undergoing tonsillectomy. In addition, VWF levels outside the adult normal range in young children may be more common than previously thought and do not necessarily predict surgical bleeding.

SUBMITTER: Gill JC

PROVIDER: S-EPMC6960478 | biostudies-literature | 2020 Jan

REPOSITORIES: biostudies-literature

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Low VWF levels in children and lack of association with bleeding in children undergoing tonsillectomy.

Gill Joan Cox JC Conley Stephen F SF Johnson Victoria P VP Christopherson Pamela A PA Haberichter Sandra L SL Diaz Christina D CD Strong Tatyana C TC Zhang Jian J Simpson Pippa P Abshire Thomas C TC Montgomery Robert R RR Flood Veronica H VH

Blood advances 20200101 1

von Willebrand disease is a common bleeding disorder, but diagnosis can be difficult in young children who have not had bleeding challenges. We sought to evaluate the correlation between bleeding and von Willebrand factor (VWF) levels in children undergoing surgical challenge with tonsillectomy. Children ages 0 to 18 undergoing tonsillectomy without a personal or family history of bleeding were enrolled prospectively following informed consent and institutional review board approval. VWF levels ...[more]

PMID: 31905240

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Project description:ImportanceBlood type (BT) O has been identified as a risk factor for bleeding complications, while non-O BTs may increase risk for thromboembolic events. Limited data are available in children undergoing tonsillectomy with or without adenoidectomy.ObjectiveTo determine whether BT O is associated with hemorrhage after tonsillectomy with or without adenoidectomy.Design, setting, and participantsRetrospective cohort study of patients younger than 22 years who underwent tonsillectomy with or without adenoidectomy at a single institution between January 1, 2008, and August 7, 2017. Statistical analysis was performed from November 2017 to January 2019.Main outcomes and measuresPrevalence of hemorrhage following surgery was defined as any bleeding requiring cauterization up to 1 month after the procedure. Data on sex, age, von Willebrand disease (VWD) status, BT, white blood cell counts, and platelet counts closest to date of surgery were collected from an electronic medical record system, and the association of these factors with hemorrhage following surgery was investigated.ResultsA total of 14 951 pediatric patients (median [range] age, 5.6 [0.8-21.9] years; 6956 [46.5%] female) underwent tonsillectomy with or without adenoidectomy. Prevalence of hemorrhage following the procedure was 3.9% (578 patients) for the full cohort and 2.8% (362 of 13 065) for patients with no BT identified or preprocedure VWD panel results at baseline. Children who had a BT identified and/or a VWD panel before surgery had higher bleeding rates (BT only, 14.9% [172 of 1156]; preprocedure VWD panel only, 4.6% [28 of 607]; and BT and preprocedure VWD panel, 13.0% [16 of 123]), all of which were significantly different from the baseline bleeding rate (P < .001). While the bleeding rates in children with BT O were not statistically different from those with non-O BT (14.8% and 14.6%, respectively; P > .99), mean von Willebrand factor values were statistically different (mean [SD] von Willebrand factor antigen level in O group, 86.9 [42.4] IU/dL in the O group vs 118.0 [53.8] IU/dL in the non-O group; P = .002; and mean [SD] von Willebrand factor ristocetin-cofactor in the O group, 72.2 [44.3] IU/dL vs 112.6 [68.0] IU/dL in the non-O group; P = .001). In addition, children older than 12 years had increased bleeding rates in the full cohort (8.3% vs 3.2%), in the testing-naive cohort (6.5% vs 2.3%), and in those with a preprocedure VWD panel only (13.5% vs 3.1%) compared with children aged 12 years or younger.Conclusions and relevanceType O blood was not a risk factor associated with hemorrhage after tonsillectomy with or without adenoidectomy despite lower baseline von Willebrand factor antigen and von Willebrand factor ristocetin-cofactor values in children with BT O vs those with non-O BT in our study cohort. No association was found between VWD status and bleeding, and there was no difference in VWD panel values in those who experienced hemorrhage vs those who did not within BT groups. Further studies elucidating the utility of von Willebrand factor values for children undergoing tonsillectomy with or without adenoidectomy are needed.

Project description:ImportanceThe rate of postoperative death in children undergoing tonsillectomy is uncertain. Mortality rates are not separately available for children at increased risk of complications, including young children (aged <3 y) and those with sleep-disordered breathing or complex chronic conditions.ObjectiveTo estimate postoperative mortality following tonsillectomy in US children, both overall and in relation to recognized risk factors for complications.Design, setting, and participantsRetrospective cohort study based on longitudinal analysis of linked records in state ambulatory surgery, inpatient, and emergency department discharge data sets distributed by the Healthcare Cost and Utilization Project for 5 states covering 2005 to 2017. Participants included 504 262 persons younger than 21 years for whom discharge records were available to link outpatient or inpatient tonsillectomy with at least 90 days of follow-up.ExposuresTonsillectomy with or without adenoidectomy.Main outcome and measuresPostoperative death within 30 days or during a surgical stay lasting more than 30 days. Modified Poisson regression with sample weighting was used to estimate postoperative mortality per 100 000 operations, both overall and in relation to age group, sleep-disordered breathing, and complex chronic conditions.ResultsThe 504 262 children in the cohort underwent a total of 505 182 tonsillectomies (median [IQR] patient age, 7 [4-12] years; 50.6% females), of which 10.1% were performed in young children, 28.9% in those with sleep-disordered breathing, and 2.8% in those with complex chronic conditions. There were 36 linked postoperative deaths, which occurred a median (IQR) of 4.5 (2-20.5) days after surgical admission, and most of which (19/36 [53%]) occurred after surgical discharge. The unadjusted mortality rate was 7.04 (95% CI, 4.97-9.98) deaths per 100 000 operations. In multivariable models, neither age younger than 3 years nor sleep-disordered breathing was significantly associated with mortality, but children with complex chronic conditions had significantly higher mortality (16 deaths/14 299 operations) than children without these conditions (20 deaths/490 883 operations) (117.22 vs 3.87 deaths per 100 000 operations; adjusted rate difference, 113.55 [95% CI, 51.45-175.64] deaths per 100 000 operations; adjusted rate ratio, 29.39 [95% CI, 13.37-64.62]). Children with complex chronic conditions accounted for 2.8% of tonsillectomies but 44% of postoperative deaths. Most deaths associated with complex chronic conditions occurred in children with neurologic/neuromuscular or congenital/genetic disorders.Conclusions and relevanceAmong children undergoing tonsillectomy, the rate of postoperative death was 7 per 100 000 operations overall and 117 per 100 000 operations among children with complex chronic conditions. These findings may inform decision-making for pediatric tonsillectomy.

Dataset Information

Low VWF levels in children and lack of association with bleeding in children undergoing tonsillectomy.

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Low VWF levels in children and lack of association with bleeding in children undergoing tonsillectomy.

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